Fatima Anis, Jijeh Abdulraouf, Alsayad Abdulsalam, Diraneyya Obayda M, Shaath Ghassan
Department of Cardiology, Division of Paediatric Cardiac Intensive Care, Ministry of National Guard - Health Affairs, Riyadh, Saudi Arabia.
King Abdullah International Medical Research Centre, Riyadh, Saudi Arabia.
Ann Pediatr Cardiol. 2025 Mar-Apr;18(2):119-123. doi: 10.4103/apc.apc_248_24. Epub 2025 Aug 29.
Absent pulmonary valve syndrome (APVS) in association with tetralogy of Fallot (TOF) is a rare congenital heart disease characterized by the aneurysmal dilatation of pulmonary arteries and associated major airway anomalies. Postoperative outcomes have been suboptimal, especially concerning dependency on positive pressure ventilation and the need for repeated cardiac reinterventions. This study aims to describe the postoperative course of these patients and to identify the risk factors associated with poor outcomes.
A retrospective review of 38 patients who underwent surgery for TOF with APVS between 2001 and 2021 at a single cardiac center was conducted. There were 16 (42%) patients in the young infants group. They required longer postoperative mechanical ventilation, 77 (interquartile ranges [IQR] 22-306) versus 17 (IQR 6-67) hours for the older children (-0.009); longer inotropic support, 127 (103-285) versus 60 (20-108) hours for older children (-0.000); longer intensive care unit (ICU) stay, 12 (4-29) versus 4 (2-7) days (-0.01), and longer hospital stay 23 (14-64) versus 15 (11-20) days (-0.02). Seven (43%) of the younger infants' group required readmission, whereas 4 (18%) of the older children needed to be readmitted ( < 0.04). Bloodstream infections (BSIs) occurred more frequently in the infants' group (31%) compared to older children (4.5%) ( < 0.03). The need for redo surgeries and the incidence of hospital-acquired infections other than BSIs did not show differences between the groups.
Age <3 months or weight <4 kg is associated with a more difficult postoperative course, characterized by longer ventilation days, a higher inotropic score, longer ICU and hospital stays, and more readmissions.
法洛四联症（TOF）合并肺动脉瓣缺如综合征（APVS）是一种罕见的先天性心脏病，其特征为肺动脉瘤样扩张及相关的主要气道异常。术后结果并不理想，尤其是在对正压通气的依赖以及需要反复进行心脏再次干预方面。本研究旨在描述这些患者的术后病程，并确定与不良预后相关的危险因素。
对2001年至2021年期间在单一心脏中心接受TOF合并APVS手术的38例患者进行回顾性研究。婴幼儿组有16例（42%）。他们术后机械通气时间更长，婴幼儿组为77（四分位间距[IQR]22 - 306）小时，大龄儿童组为17（IQR 6 - 67）小时（-0.009）；强心支持时间更长，婴幼儿组为127（103 - 285）小时，大龄儿童组为60（20 - 108）小时（-0.000）；重症监护病房（ICU）住院时间更长，婴幼儿组为12（4 - 29）天，大龄儿童组为4（2 - 7）天（-0.01），住院时间更长，婴幼儿组为23（14 - 64）天，大龄儿童组为15（11 - 20）天（-0.02）。婴幼儿组中有7例（43%）需要再次入院，而大龄儿童组中有4例（18%）需要再次入院（<0.04）。与大龄儿童组（4.5%）相比，婴幼儿组血流感染（BSIs）发生率更高（31%）（<0.03）。再次手术的需求以及除BSIs之外的医院获得性感染发生率在两组之间未显示出差异。
年龄<3个月或体重<4kg与更困难的术后病程相关，其特征为通气天数更长、强心评分更高、ICU和住院时间更长以及再次入院更多。
